Part of “CHAPTER 92 – PRECOCIOUS AND DELAYED PUBERTY“

Prolonged exposure to estrogens or androgens from a peripheral source may lead to early initiation of gonadotropin-dependent PP. Patients with virilizing adrenal tumors, uncontrolled or undiagnosed CAH, McCune-Albright syndrome, familial male PP, and ovarian tumors have been reported to progress to gonadotropin-dependent PP.48,57,69,70 The mechanism seems to be secondary to inappropriate maturation of the hypothalamic– pituitary–gonadal axis after prolonged sex steroid exposure. The degree of bone age advancement has also been thought to correlate with the timing of the development of central puberty, as most reported cases have had bone ages of at least 10.5 years at the time of activation of the hypothalamic–pituitary–gonadal axis. However, a 5-year-old girl with a history of an ovarian tumor at the age of 7 months developed gonadotropin-dependent PP at a bone age of only 5.5 years.71 Central activation of the hypothalamic–pituitary–gonadal axis usually occurs after the primary problem has been brought under control and sex steroid levels are lowered, relieving the hypothalamic–pituitary
axis of negative feedback. Normal gonadotropin secretion then overrides the autonomous hormone production seen in McCune-Albright syndrome and familial male PP.48,57